3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate

 化学構造式
31023-24-2
CAS番号.
31023-24-2
化学名:
别名:
英語名:
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
英語别名:
Isovalerylcarnitine;Isovaleroyl Carnitine;O-isovalerylcarnitine;ISOVALERYL-L-CARNITINE;3-methylbutyrylcarnitine;Isovaleryl-L-carnitine >=97.0% (TLC);3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate;(3R)-3-(3-Methylbutanoyloxy)-4-(trimethylazaniumyl)butanoate;(R)-3-((3-methylbutanoyl)oxy)-4-(trimethylammonio)butanoate(WXG00321);(R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt
CBNumber:
CB11236041
化学式:
C12H23NO4
分子量:
245.32
MOL File:
31023-24-2.mol
MSDS File:
SDS

3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate 物理性質

貯蔵温度 :
2-8°C
溶解性:
Soluble in DMSO
外見 :
Solid
色:
White to off-white
光学活性 (optical activity):
[α]/D -23±2°, c = 1 in H2O
BRN :
5946880

安全性情報

3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate 価格 もっと(4)

メーカー 製品番号 製品説明 CAS番号 包装 価格 更新時間 購入
Sigma-Aldrich Japan 51371 ≥97.0% (TLC)
Isovaleryl-L-carnitine ≥97.0% (TLC)
31023-24-2 10mg ¥11200 2024-03-01 購入
Sigma-Aldrich Japan 51371 ≥97.0% (TLC)
Isovaleryl-L-carnitine ≥97.0% (TLC)
31023-24-2 50mg ¥42200 2024-03-01 購入
Sigma-Aldrich Japan 91403 analytical standard
Isovaleryl-L-carnitine analytical standard
31023-24-2 10mg ¥18600 2021-03-23 購入
Sigma-Aldrich Japan 91403 analytical standard
Isovaleryl-L-carnitine analytical standard
31023-24-2 50mg ¥60400 2021-03-23 購入

3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate 化学特性,用途語,生産方法

使用

Isovaleryl-L-carnitine may be used as an analytical reference standard for the quantification of the analyte in biological samples using chromatography-based techniques.

一般的な説明

Isovaleryl-L-carnitine belongs to the acylcarnitines class of compounds. Acyl-L-carnitines are mostly produced from L-carnitine, which plays an important role in fatty acid metabolic pathways. The functions and roles of acyl-L-carnitines in various tissues like brain, heart, and muscle continue to attract much interest, since a number of diseases caused by defects of mitochondrial transport are characterized by specific metabolic dysfunctions and depend on the physiological role of the affected carrier in intermediary metabolism.

Biochem/physiol Actions

Isovalerylcarnitine is the phenotypic abnormality in iso-valeric acidemia resulting from an accumulation of isovaleric acid, which is toxic to the central nervous system. Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. An alternative pathway through glycine-N-acylase allows detoxification by producing isovaleryl-glycine, which is excreted. Thus, isovalerylcarnitine and isovalerylglycine are the hallmarks of this disorder in plasma and urine, respectively, and are elevated regardless of a patient′s metabolic condition.

3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate 上流と下流の製品情報

原材料

準備製品


3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate 生産企業

Global( 24)Suppliers
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Energy Chemical 021-58432009 400-005-6266
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  • 31023-24-2
  • 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
  • Isovalerylcarnitine
  • 3-methylbutyrylcarnitine
  • Isovaleroyl Carnitine
  • (3R)-3-(3-Methylbutanoyloxy)-4-(trimethylazaniumyl)butanoate
  • (R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt
  • ISOVALERYL-L-CARNITINE
  • O-isovalerylcarnitine
  • Isovaleryl-L-carnitine >=97.0% (TLC)
  • (R)-3-((3-methylbutanoyl)oxy)-4-(trimethylammonio)butanoate(WXG00321)
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